Top 5 Most-Read Pulmonary Fibrosis Content of 2025

From artificial intelligence–powered imaging to long-awaited therapeutic breakthroughs, 2025 marked a pivotal year in pulmonary fibrosis research and care. Readers gravitated toward stories highlighting both innovation in how disease progression is measured and major advances in treatment, including compelling evidence for combination therapy in complex disease and the FDA approval of the first novel idiopathic pulmonary fibrosis (IPF) drug in more than a decade.

These articles reflect growing momentum in the field, with new tools and therapies offering renewed hope for improving outcomes in IPF and progressive pulmonary fibrosis (PPF).

Here are the top 5 most-read pulmonary fibrosis articles of 2025.

5. Deep Learning Biomarkers Help Uncover ENV-101 Treatment Effects in IPF

An AI-based analysis of high-resolution computed tomography (CT) scans showed that patients with IPF treated with the Hedgehog pathway inhibitor ENV-101 had early improvements in lung volume and signs of reduced fibrosis in a phase 2a trial presented at the American Thoracic Society (ATS) 2025 International Conference. Over 12 weeks, ENV-101 was associated with a significant increase in lung volume and improvements in percent predicted forced vital capacity (FVC) compared with placebo, along with a significant reduction in pulmonary vascular volume and a trend toward less fibrosis.

These imaging findings closely correlated with lung function measures, suggesting deep learning–based CT tools can sensitively detect clinically meaningful treatment responses. While limited by small sample size and short follow-up, the results support ENV-101’s potential benefit in IPF and the use of AI imaging as a complementary trial end point.

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4. Predicting the Course of Progressive Pulmonary Fibrosis With FOT

The forced oscillation technique (FOT) may help identify patients with PPF, a worsening subtype of interstitial lung disease (ILD) that can be difficult to assess using standard pulmonary function tests, according to a retrospective study. Analyzing data from 160 patients with fibrosing ILDs, researchers found that patients with PPF had significantly different inspiratory reactance at 5 Hz (Xrs5) compared with those with stable non-IPF ILDs, and that Xrs5 showed moderate correlations with FVC and diffusing capacity for carbon monoxide.

While conventional pulmonary function tests remained superior overall, the authors concluded that combining them with FOT, an easy, noninvasive technique requiring minimal patient cooperation, could improve early identification of progressive disease, particularly in patients unable to perform traditional testing, though prospective studies are needed to confirm its clinical utility.

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3. Dual Therapy Appears to Benefit Patients With Pulmonary Fibrosis, Pulmonary Hypertension

Patients with pulmonary fibrosis and pulmonary hypertension who received pulmonary vasodilator therapy in addition to antifibrotic treatment had significantly better transplant-free survival than those treated with antifibrotics alone, researchers found. Among 155 patients with right-heart catheterization–confirmed pulmonary hypertension, those receiving combination therapy showed markedly higher transplant-free survival and longer survival from the time of catheterization, despite having more severe pulmonary hypertension at baseline.

Although improvements in exercise capacity did not reach statistical significance, the findings provide some of the first direct evidence supporting dual therapy in this population and suggest a potential pulmonary vascular phenotype in pulmonary fibrosis, while underscoring the need for larger prospective trials to confirm these benefits.

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2. FDA Approves Nerandomilast for Idiopathic Pulmonary Fibrosis

The FDA approved nerandomilast (Jascayd), a phosphodiesterase 4B inhibitor from Boehringer Ingelheim, for the treatment of adults with IPF, marking the first novel IPF therapy approved in more than a decade. The approval was based on results from the phase 3 FIBRONEER-IPF trial and a supportive phase 2 study, which together showed that nerandomilast significantly slowed decline in FVC compared with placebo, including in patients with prior antifibrotic use.

In FIBRONEER-IPF, adjusted FVC decline at 52 weeks was substantially lower with nerandomilast than placebo, and pooled data presented at ERS 2025 suggested a survival benefit in patients not receiving background therapy. The drug was generally well tolerated, with diarrhea the most common adverse event and fewer safety concerns than existing antifibrotics, leading experts to suggest nerandomilast could become a first-line treatment option for IPF.

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1. Nerandomilast Improves Lung Function in Progressive Pulmonary Fibrosis: FIBRONEER-ILD

Nerandomilast met its primary end point in the phase 3 FIBRONEER-ILD trial, showing a significant improvement in FVC compared with placebo in patients with PPF. The double-blind trial enrolled 1178 patients across more than 40 countries, with participants receiving 9 mg or 18 mg of nerandomilast twice daily for at least 52 weeks. The safety and tolerability of nerandomilast were consistent with prior phase 2 and FIBRONEER-IPF trial results, and adverse events were comparable with placebo. Boehringer Ingelheim has submitted a new drug application to the FDA and other authorities based on these findings.

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